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Case Reports
. 2020;80(1):81-83.

[Ileitis as presentation of lymphoma in Wiskott-Aldrich syndrome]

[Article in Spanish]
M Silvina Odstrcil-Bobillo  1   2 , Dana Kohan  3 , Paula G Heller  4   5 , Victoria Otero  1   2 , María Paula Russo  1 , Ana L Basquiera  1   2
Affiliations
  • PMID: 32044744
Free article
Case Reports

[Ileitis as presentation of lymphoma in Wiskott-Aldrich syndrome]

[Article in Spanish]
M Silvina Odstrcil-Bobillo et al. Medicina (B Aires). 2020.
Free article

Abstract
in English, Spanish

Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.

El síndrome de Wiskott-Aldrich (SWA) es un raro síndrome de inmunodeficiencia primaria ligado al cromosoma X que se asocia con aumento de incidencia de infecciones, trastornos autoinmunes y neoplasias. Se presenta el caso de un varón de 41 años con diagnóstico de síndrome de Wiskott-Aldrich y cuadro de ileítis como forma de presentación de un síndrome linfoproliferativo. La ileítis, en el contexto del paciente, representa un problema clínico dado el gran número de diagnósticos diferenciales (enfermedad inflamatoria intestinal, infecciones, neoplasias y enfermedades linfoproliferativas) por lo que suele requerir diagnóstico anatomopatológico y consideraciones particulares respecto al posterior tratamiento específico.

Keywords: Wiskott-Aldrich; ileitis; lymphoproliferative syndrome.

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